Intrahepatic bile ducts (IHBDs) develop from bipotential liver progenitor cells in contact with the mesenchyme of the portal vein and thus form the ductal plates.

The ductal plates are remodeled into mature tubular ducts. Lack of remodeling results in the persistence of periportal epithelial sleeves or "ductal plate malformation" (DPM).

A proposal is that virtually all congenital diseases of IHBDs represent examples of ductal plates malformations (DPM).

Classification

 autosomal recessive polycystic kidney disease (hepatic ARPKD) (50% of children, 70% of families)

• DPM of interlobular bile ducts associated with tubular dilatation of collecting renal tubules

 Caroli disease > DPM of the larger IHBDs
 Caroli syndrome > Caroli disease + congenital hepatic fibrosis
 von Meyenburg complexes > DPM of smaller interlobular ducts (liver cysts in autosomal dominant polycystic kidney disease)
 mesenchymal hamartoma
 Meckel syndrome
 non-syndromal ductal plate malformation

References

 Sergi C, Adam S, Kahl P, Otto HF. Study of the malformation of ductal plate of the liver in Meckel syndrome and review of other syndromes presenting with this anomaly. Pediatr Dev Pathol. 2000 Nov-Dec;3(6):568-83. PMID: #11000335#